Course Authors

Martin J. Carey, M.D.

Dr. Carey reports no commercial conflict of interest.

Estimated course time: 1 hour(s).

Albert Einstein College of Medicine – Montefiore Medical Center designates this enduring material activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

In support of improving patient care, this activity has been planned and implemented by Albert Einstein College of Medicine-Montefiore Medical Center and InterMDnet. Albert Einstein College of Medicine – Montefiore Medical Center is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC), to provide continuing education for the healthcare team.

 

Learning Objectives

Upon completion of this Cyberounds^®, you should be able to:

• Discuss the classical presentation to the emergency department of four pediatric conditions

• List the differential diagnoses for these conditions

• Discuss emergency department management, including pharmaceutical therapy and disposition, for each condition.

 

Patient One

A four-year-old boy presents to the emergency department at 10:00 PM. He usually enjoyed excellent health. He was a full term, normal delivery infant and had never been hospitalized. He was up-to-date on all immunizations and other than very occasional ear infections had 'never been ill.'

His mother states that he has been unwell for about ten days. They had seen a primary care physician and the child had been on antibiotics for the past six days. However, despite this, the child remained pyrexial. Tonight, the fever was 39ºC. The child was irritable and off his food. The mother had noted that he had 'red eyes', and this seemed to be getting a little worse. The mother commented that light seemed to hurt her child's eyes.

The mother also commented that her child's lips were cracked and dry, and that the tongue seemed to be 'quite red'. The presentation to the emergency department had been triggered by the return home from work of the mother to find that the child had developed swelling of the hands and feet. The hands had also seemed to turn red.

Q. From the history, what condition comes to mind? What are you going to look for carefully in the examination?

A. From the history alone, the emergency physician would have to be very concerned that this child may have Kawasaki's Disease, also known as mucocutaneous lymph node syndrome. In order to be more certain of the diagnosis, the examination should be conducted to:

1. Eliminate other causes of fever
2. Examine the skin carefully for evidence of a rash
3. Examine the neck for evidence of lymphadenopathy, particularly in the anterior cervical nodes overlying the sternocleidomastoid. All lymph node groups should be examined and the spleen carefully palpated. Presence of widespread lymphadenopathy or splenomegaly should trigger the search for an alternative diagnosis.

The diagnosis of Kawasaki's Disease involves the following:

1. Presence of fever (>38.5ºC) for more than five days unresponsive to antibiotics
2. The presence of at least four of the following five clinical findings:
   1. Bilateral conjunctival injection
   2. Oropharyngeal mucous membrane changes, including reddened or fissured lips, strawberry tongue and/or injected palate
   3. Peripheral extremity changes, including erythema or edema of the hands or feet and/or desquamation around the nails
   4. Polymorphous, non vesicular, rash, which is primarily truncal
   5. Cervical lymphadenopathy, at least 1.5 cm

Although these criteria are neither 100% sensitive nor specific, a child who has so many signs of the disease should be regarded as having the disease. The pitfall comes in that, occasionally, children will have the disease but not exhibit all of the criteria. A high index of suspicion should be held for this disease.

Our patient did indeed exhibit cervical lymphadenopathy and he also had a rash over the trunk, though that was fading.

The differential diagnosis for Kawasaki's Disease must include some of the exanthematous diseases of childhood, such as measles, and adenovirus. These conditions classically lack the limb edema of Kawasaki's. Other conditions to be considered include toxic shock syndrome (usually no cervical lymphadenopathy and also seen usually in older children), scarlet fever (usually pharyngitis and more widespread cervical lymphadenopathy, no eye signs) and Stevens-Johnson syndrome (no limb involvement).

Q. What is the most feared complication of Kawasaki's Disease?

A. The most feared complication relates to the development of cardiovascular problems. These can include pericardial effusions, congestive heart failure and coronary artery aneurysms. A pancarditis can occur in the early phases of the disease. Coronary artery aneurysms occur in 15-25% of untreated children and can lead to myocardial infarction, chronic coronary artery insufficiency or sudden death. The overall mortality from Kawasaki's disease is 0.4%. Treatment reduces the incidence of coronary artery aneurysms by three- to five-fold, especially the giant aneurysms that are most often responsible for death. Treatment needs to be started within 10 days to be most effective.

Q. What are the treatment options for this child?

A. The treatment of choice for children with Kawasaki's Disease is intravenous immunoglobulin. As noted above, treatment within 10 days of disease onset significantly reduces disease duration and minimizes the incidence of complications. Treatment regimens vary, but a single large infusion (2 g/kg) of immunoglobulin is given over 8-12 hours. Although theoretical risks are present with the use of intravenous immunoglobulin (such as transmission of blood borne pathogens, for example, Hepatitis C) the risks are far outweighed by the benefits.

Aspirin and other non-steroidal anti-inflammatory agents can be used with immunoglobulin to aid in patient comfort. Aspirin was the first agent used in the management of Kawasaki's Disease. It is still used, in some cases in a high dose regimen (80 mg/kg/day), while others use a lower dose regimen at 30 mg/kg/day. There is no definite evidence that aspirin alone affects the development of coronary artery aneurysms.

All children with a potential diagnosis of Kawasaki's Disease should be admitted to hospital and the assistance of a pediatrician familiar with the disease sought.

The annual incidence of the disease in the United States is between 4 and 15 cases per 100,000 children aged less than five. It is more common in males. The cause is unknown but an infectious agent is suspected.

Patient Two

It is spring. You arrive at work and your first patient is an eight-year-old boy. His father notes that his son has abdominal pain. Certainly, the child seems to be in some discomfort. He is lying on the bed and seems to have a spasmodic abdominal pain. History, taken from his father, reveals that the child was born at term by cesarean section (for presumed cephalopelvic disproportion). He has never been hospitalized, is up-to-date for his immunizations and is on no medications. He occasionally gets 'ear infections' and had experienced a 'viral throat infection' some seven to 10 days earlier, which seemed to settle with over-the-counter remedies. The father notes that the child has been unwell for a couple of days. He has complained of 'aches' in his knees and ankles. He vomited a couple of times overnight and had experienced increasing abdominal pain. There had been no other symptoms known by the father.

Examination reveals a white child who appears to have colicky abdominal pain. He is hemodynamically stable and is apyrexial. Cardiovascular and respiratory system examination reveals no abnormality; the abdomen is generally tender, but without rebound. Bowel sounds are present. Testes are descended and non-tender. Knees and ankles are slightly swollen and tender to move. You note a rash over the extensor surfaces of the lower limbs and, on turning the child, note that it is also present over the buttocks. The rash appears purpuric and is palpable.

Q. What is the diagnosis here?

A. The child most likely has Henoch-Schönlein purpura. The combination of abdominal pain, arthralgia and palpable purpura over the lower limbs is classical. This disease seems to occur more frequently in the winter and spring, affects males more than females and the children are usually under the age of ten years. The condition can affect any age and is well described in adults, though rare.

The criteria for making the diagnosis are the presence of two of the following four:

• Age less than 20 years at onset
• Palpable purpura
• Diffuse abdominal pain
• Biopsy evidence of granulocytes in the walls of arterioles or venules

The differential diagnosis of this condition includes other vasculitic conditions, such as Wegener's granulomatosis, systemic lupus erythematosus and microscopic polyarteritis. These conditions are usually only considered if the patient has an atypical rash or in the time after the onset of joint pains (but before the rash develops). Other causes of purpura, such as sepsis and thrombocytopenia, can be excluded by the clinical picture or by basic hematological investigations.

Q. What is the most feared complication of this condition?

A. The complication most feared with Henoch-Schönlein purpura is renal disease. Renal disease occurs in about 40% of cases overall (the reported range is from 20 to 100%). The renal disease may be nephritis or a true nephritic syndrome. Nephritis is manifest by microscopic hematuria in most cases, though up to 20% of patients with renal disease will have gross hematuria. Renal disease usually occurs within four weeks of the initial symptoms. In some cases, renal disease may be a late manifestation.

Other complications that can occur rarely with Henoch-Schönlein purpura include cerebral manifestations (headaches, seizures, paresis and coma), testicular pain (which must be distinguished from torsion of the testis), cholecystitis and myocardial infarction.

Q. What therapy would you institute in the emergency department and what disposition would you arrange? What should you be sure to discuss with the relatives about this condition?

A. Usually, Henoch-Schönlein purpura is a self-limiting illness and no therapy is indicated. Steroids have been used in the acute phase (1 mg/kg/day) with some success to manage the joint pains and the abdominal pain. There is some debate as to whether steroids influence the development or the course of the renal disease. Steroids do appear to improve outcome in patients with severe renal disease. If renal disease is rapidly progressive, some have suggested plasma exchange. However, there are no controlled clinical trials to evaluate efficacy. Other agents that have been used include dapsone and Factor XIII replacement. Non-steroidal agents have not been shown to be effective, and should probably be avoided, especially if the patient has renal disease.

If the abdominal pain can be controlled and there is no evidence of active renal disease, the patient could be discharged to home, provided there are adequate arrangements for close follow-up. Severe arthritis, abdominal pain or renal disease should probably lead the physician to consider admission.

The emergency department physician should warn the patient and their patients that the disease can recur, with arthritis and abdominal pain, over the weeks after initial presentation. About one-third of all patients will have at least one recurrence.

The disease is more common in boys. It is an acute leukocytoclastic vasculitis. The exact etiology is unknown. It is known that IgA plays a key role in the disease, with a raised serum level, IgA containing immune complexes and deposition of IgA in the walls of blood vessels.

Patient Three

A 10-month-old male child presents to the emergency department in the arms of his mother. The mother states that the child has been vomiting and unable to keep his feeds down, is reluctant to drink and seems to be in pain. She describes him as 'sleepier than usual.' He was born at term and has no significant history to date. The presentation was triggered by a single episode tonight of blood streaked in the stool. Examination reveals an unhappy child, who is apyrexial with a pulse of 125. Cardiovascular and respiratory examination is unremarkable, as is examination of the head and neck. The child does appear to be about 5% dehydrated. Abdominal examination is also unremarkable with no masses felt. The stool, which has been brought in by the mother, is guaiac positive.

Q. What condition are you highly suspicious of at this point?

A. With this history, you should be highly suspicious that this child has an intussusception. Although the classic presentation includes abdominal pain, vomiting, bloody stool and abdominal mass, only about 50% of patients present with all four of these signs. The condition is more common in males than females and is generally seen in children under the age of 12 months. The patient is usually lethargic. An intussusception is where one part of the bowel telescopes into another. There are four types of intussusception described:

• Ileo-colic (the commonest)
• Ileo-ileal
• Ileoileocolic
• Colocolic

Initially the blood supply to the intussusception (the part that goes into the bowel) is compressed. Venous return is impeded, and swelling and engorgement of the bowel wall result. Eventually, arterial compromise causes total ischaemia of the bowel wall. If not treated, bowel necrosis and perforation can result. The bleeding is a result of bowel ischaemia.

Q. Given your suspicions for intussusception, what is the next step in your investigation?

A. The next step in investigating this child is either an air or contrast enema or an ultrasound. Classically, water-soluble contrast (Gastrografin^®) has been used.

Q. Over the past couple of years, there was an increase in the number of cases of intussusception in the United States. This increased risk has now disappeared. Why?

A. The increased risk was associated with the use of the rotavirus vaccine, which has now been withdrawn in the United States. The risk of developing intussusception was of the order of 1 in 5,000 vaccinations and was greatest within three to seven days of the first injection (which was given at three months of age). There was a slight increase in risk of intussusception after the second shot and no increase after the third. Although this risk was considered too high for the United States, where rotavirus is not usually a life threatening disease, this may not be the case in developing countries, where the vaccine may continue to be used. Physicians practicing in countries where the vaccine is still in use should be aware of this risk.

Other causes of intussusception include lymphoma, Meckel's diverticulum, Henöch-Schonlein purpura, cystic fibrosis, celiac disease and after surgery (either from an appendiceal stump, or a suture line).

Q. What are your treatment options?

A. If the patient is acutely unwell, with hypotension, perforation or peritonitis, then emergent surgical intervention is indicated. Even if the patient appears well, surgical opinion should be sought at an early stage. An intravenous infusion and a nasogastric tube are generally required.

Reduction by barium enema has been a standard of practice for some time in the U.S. and Europe. Recently, experience has been gained using the controlled insufflation of air -- success rates at least equivalent to that of enema reductions have been reported. Generally, barium enema reduction is effective in 50-60% of cases, with a 10% spontaneous reduction rate and a 10% recurrence rate. Morphine sedation is often employed. Great care should be taken with enema reduction to avoid complications such as perforation. The enema column should not be more than three feet above the patient and manual palpation of the abdomen is contraindicated during the procedure.

If enema reduction is not achieved, or if there are signs as noted above, or if there is a definite lead point (resulting from the increased rate of recurrence in these cases), surgery is required. (A 'lead point' is an area of bowel that is thought to act as the trigger for the development of the intussusception. It may be caused by lymphatic enlargement within the bowel wall or, sometimes, a Meckel's Diverticulum may act as a lead point.)

Probably, all children with this diagnosis should be admitted for observation, even if enema reduction is possible. Many recommend re-examination after 24 hours to check for recurrence.

Patient Four

You are called to see a four-year-old girl who has been brought by her mother to the emergency department because of a swelling of the left wrist. The mother had noted the swelling when the child awoke that morning. The mother noted that the child had been unwell over the past two weeks with a low-grade fever, a cough and complaints of a sore throat. She had seen the primary care doctor two days earlier because of swelling in the child's right knee. No definite diagnosis was made at that time. The child was usually in good health, but had not had any immunizations. The single mother admitted to smoking 40 cigarettes daily. There was another child at home, who had also experienced a sore throat. On examination, she was an unkempt child with a mild cough. She had a low-grade fever at 39ºC orally. Pulse was regular at 90 beats per minute. Examination of the oropharynx revealed large tonsils with exudate present. Examination of the cardiovascular system revealed a soft systolic murmur, heard best in the apical region. The mother did not know if this had been present previously. Respiratory system examination was unremarkable. Both the wrist and the knee were warm, slightly red and swollen.

Q. What diagnosis are you suspicious of and what is the cause?

A. With the combination of sore throat, fever, joint involvement and a cardiac murmur, you would have to be suspicious that this child has rheumatic fever. The cause of rheumatic fever is Group A streptococcal infection. However, not all Group A streptococci cause rheumatic fever. For example, although Group A streptococcus can cause soft tissue infections and other extra pharyngeal infections, these types are never considered to have caused rheumatic fever. Certain strains are considered to be 'rheumatogenic' and these are types that consistently cause pharyngitis. Group A streptococcus can be serotyped utilizing so-called 'M' serotypes. The most common serotype that is associated with rheumatic fever is M5.

Q. There are criteria to assist in making the diagnosis of rheumatic fever. What name is given to these criteria, and what are the major and minor criteria?

A. The criteria are the 'Jones' criteria. These were initially described in 1944, and have been revised and modified on three occasions since. The diagnosis of rheumatic fever requires the presence of evidence for a previous streptococcal infection, plus at least one major and two minor criteria, OR two major criteria.

Major Criteria

• Carditis. Occurs in about a third of cases. Frequently seen in children.
• Polyarthritis. Occurs in majority of new cases (65-75%). Affects lower limbs more commonly.
• Chorea. Occurs in about 10% of cases. Purposeless movements of the limbs, which increase with anxiety and disappear with sleep are characteristic.
• Erythema marginatum. Rare finding -- less that 5%.
• Subcutaneous nodules. Occur on extensor surface of limbs. Occurs in 20%.

Minor Criteria

• Arthralgias
• Fever
• Increased erythrocyte sedimentation rate or C-reactive protein
• Prolonged PR interval
• Previous rheumatic fever
• Increased antistreptolysin O titre

Thus, in our case, it would appear that if we can determine that the pharyngitis was caused by a streptococcal infection, then our patient may well have two major (polyarthritis and carditis) and one minor (fever) criteria.